Journal of Mycosis Fungoides

7/27/2019 Journal of Mycosis Fungoides

1/4

Journal of Cosmetics, Dermatological Sciences and Applications, 2012, 2, 64-67doi:10.4236/jcdsa.2012.22015 Published Online June 2012 (http://www.SciRP.org/journal/jcdsa)

Hypopigmented Mycosis Fungoides in a 7-Year-Old Boy

Felipe Nazareth*

, Maria Victoria Quaresma, Fred Bernardes, Carlos Gustavo Carneiro Castro,Edilbert Pellegrini Nahn, Jose Augusto da Costa Nery, Mayra Carrijo Rochael, Omar Lupi

Department of Dermatology, Policlnica Geral do Rio de Janeiro, Rio de Janeiro, Brazil.Email: *[email protected]

Received December 22nd,2011; revised January 26th, 2012; accepted February 9th, 2012

ABSTRACT

Hypopigmented mycosis fungoides (HMF) is an uncommon variant of cutaneous T-cell lymphoma. It is more frequentin dark-skinned people, particularly children. The HMF diagnose is difficult, especially in early stages because this con-dition resembles benign skin diseases. Thus is histopathological analysis very important for the diagnosis. We report a

case of a 7-year-old child with widespread HMF confirmed by histopathology that showed cells tagging along the der-mal/epidermal junction and extending into the epidermis in a pattern of epidermotropism and focal cell aggregates inthe epidermis (Pautriers microabscess). We demonstrate the importance of clinical suspicion for this cutaneous neopla-sia in patients with hypopigmentated lesions.

Keywords: Hypopigmented; Child; Mycosis Fungoides

1. Introduction

Mycosis fungoides (MF), the most common type of cu-

taneous T-cell lymphoma is more frequent in the elderly.

Although it is rare in childhood and adolescence, there

has been an increased recognition that MF may arise in

children and young adults [1,2]. Diagnosis is difficult inthe early stage of the disease because it mimics several

benign skin disorders including eczema, psoriasis, and

contact dermatitis. Multiple biopsies may be necessary to

confirm the diagnosis [1]. The most common findings are

erythematous, scaly, and hypopigmented macules [3].

The etiology of MF remains unknown but various theo-

ries have been postulated, such as chronic antigenic

stimulation, atopy and, occupational, environmental, and

viral exposures [1,4]. We report a case of hypopigmented

mycosis fungoides (HMF) in a dark-skinned 7-year-old

boy.

2. Case Report

A 7-year-old boy presented with a 1-year and 5 months

history of erythematous patches which turned into hy-

popigmentation (Figures 1 and 2). The first patch was

noticed on the left arm after intense sun exposure. The

patient had no other significant medical data and there

was no use of any medication. On skin examination,

multiple hypopigmented and oval macules and patches

were observed on the trunk, face and limbs covering

more than 10% of the skin. The lesions were accom-

Figure 1. A 7-year-old boy presenting with hypopigmented

macules on limbs, trunk and abdomen.

panied by discrete pruritus and the skin sensibility on the

patches was normal. General physical examination re-*Corresponding author.

Copyright 2012 SciRes. JCDSA


2/4

Hypopigmented Mycosis Fungoides in a 7-Year-Old Boy 65

(a)

(b)

Figure 2. (a) Generalized hypopigmented macules covering

over 10% of the body surface area; (b) Hypopigmented ma-

cules/patches on trunk e superior right limb.

vealed no lymphadenopathy or hepatosplenomegaly. The

hypopigmented patchs biopsy on the abdomen revealed

cells tagging along the dermal/epidermal junction and

extending into the epidermis in a pattern of epidermotro-

pism. Some of these cells had perinuclear halos and focal

aggregates were noticed in the epidermis (Pautriers mi-

croabscess). Immunophenotypical analysis revealed that

neoplastic cells were CD3 positive, which is a pan T-cell

marker. The lymphocytes were mostly of the T helper

CD4 positive (90%) phenotype but T suppressor/cyto-

toxic CD8 positive cells were also detected in a small

proportion (10%). Complete blood cell count with exami-

nation for Sezary cells, chest radiography and abdominalultrasonography did not show any abnormalities. A diag-

nosis of stage 1B [5] hypopigmented mycosis fungoides

was established based on clinical and histopathologi-

cal/immunophenotypical findings. Therefore narrowband

UVB therapy three times a week was planned (Figures 3

and4).

3. Discussion

HMF is an atypical, rare and unique variant of MF cha-

racterized by solely hypopigmented patches or more com-

monly in combination with erythematous patches or

plaques, usually observed in dark skinned individuals,

and exceedingly rare in Caucasians and others with fair

skin types [6,7].

The mechanism of the hypopigmentation in this MF

variant is still unclear. It has been postulated that atypical

lymphoid cells infiltrating the epidermis cause melano-

cyte degeneration and abnormal melanogenesis as a re-

sult of non-specific cell injury [8]. In contrast, other au-

thors proposed that the loss of pigment results from a

defect in melanosome transfer from melanocyte to kerati-

nocytes [9,10].

The epidermal infiltrate in hypopigmented MF is re-

ported to be predominantly of CD8-positive lymphocytes[11,12], however, our patient had an epidermal infiltrate

with mostly CD4-positive lymphocytes.

HMF should be included in the differential diagnosis

of any persistent hypopigmented macule or patch that is

resistant to treatment. It is emphasized that this variant

Figure 3. Histopathological analysis showing on high-power

iew a lymphocytic infiltrate in the epidermis.v



3/4

Hypopigmented Mycosis Fungoides in a 7-Year-Old Boy


66

(a) (b)

(c) (d)

Figure 4. Immunohistoquemical stanning showing high positivity to CD3 (a) and to CD4 (b), whereas CD8 (c) and CD7 (d)

present lower positivity.

REFERENCESusually presents at a younger age than the classical MF.Sequential biopsies are recommended if the diagnosis

remains in doubt. Although the majority of cases have a

benign course we concluded that it is essential to stage

this disease clinically since occasional cases had an un-favorable progression. Furthermore, follow up of the pa-

tient is clearly indicated since recurrence is common and

some cases behave aggressively [6,10].

[1] N. Onsun, Y. Kural, O. Su, et al., Hypopigmented My-cosis Fungoides Associated with Atopy in Two Chil-

dren, Pediatric Dermatology, Vol. 23, No. 5, 2006, pp.

493-496.[2] J. J. Crowly, A. Nikko, A. Varghese, et al., Mycosis

Fungoides in Young Patients: Clinical Characteristics and

Outcome,Journal of the American Academy of Derma-tology, Vol. 38, No. 5, 1998, pp. 696-701.

doi:10.1016/S0190-9622(98)70198-7 Long follow-ups have demonstrated that hypopig-

mented MF responds well to UVA and UVB, or topical

mechlorethamine, resulting in remission for years [12].

We decided to initiate UVB narrow band therapy for our

patient after staging his disease (stage 1B) since we be-

lieve that this option is a safe one in this age and also

because it has been shown to be effective to treat this

disease [1].

[3] S. E. Whitmore, et al., Hypopigmented Mycosis Fun-goides,Archives of Dermatology, Vol. 130, No. 4, 1994,

pp. 476-480.

doi:10.1001/archderm.1994.01690040080012

[4] E. Tuyp, A. Burgoyne, T. Aitchison, et al., A Case-Control Study of Possible Causative Factors in Mycosis

Fungoides, Archives of Dermatology, Vol. 123, No. 2,
http://dx.doi.org/10.1016/S0190-9622(98)70198-7http://dx.doi.org/10.1016/S0190-9622(98)70198-7http://dx.doi.org/10.1001/archderm.1994.01690040080012http://dx.doi.org/10.1001/archderm.1994.01690040080012http://dx.doi.org/10.1001/archderm.1994.01690040080012http://dx.doi.org/10.1016/S0190-9622(98)70198-7


4/4

Hypopigmented Mycosis Fungoides in a 7-Year-Old Boy 67

1987, pp. 196-200. doi:10.1001/archderm.123.2.196

[5] Y. H. Kim, et al., Long-Term Outcome of 525 Patientswith Mycosis Fungoides and Szary Syndrome,Archives

of Dermatology, Vol. 139, No. 7, 2003, pp. 857-866.

[6] A. Di Landro, L. Marchesi, L. Naldi, T. Motta and T.Cainelli, A Case of Hypopigmented Mycosis Fungoidesin a Young Caucasian Boy, Pediatric Dermatology, Vol.

14, No. 6, 1997, pp. 449-452.

doi:10.1111/j.1525-1470.1997.tb00687.x

[7] E. Sezer, T. Sezer, A. Senayli, D. Koseoglu and N. Filiz,Hypopigmented Mycosis Fungoides in a Caucasian

Child,European Journal of Dermatology, Vol. 16, No. 5,

2006, p. 584.

[8] S. M. Breathnach, P. H. Mckee and N. P. Smith, Hy-popigmented Mycosis Fungoides: Report of Five Cases

with Ultrastructural Observations, British Journal of

Dermatology, Vol. 106, No. 6, 1982, pp. 643-649.

doi:10.1111/j.1365-2133.1982.tb11678.x

[9] D. J. Goldberg, R. A. Schinella and P. Kechijian, Hy-popigmented Mycosis Fungoides: Speculations about theMechanism of Hypopigmentation, The American Jour-nal of Dermatopathology, Vol. 8, No. 4, 1986, pp. 326-330.doi:10.1097/00000372-198608000-00009

[10]

J. T. Al-Ratrout, M. Al-Nazer andN. A. Ansari, Hy-popigmented Mycosis Fungoides in a Twenty-Year-OldSaudi Woman with Fair Skin, Indian Journal of Derma-tology, Vol. 51, No. 2, 2006, pp. 115-117.doi:10.4103/0019-5154.26932

[11] L. El-Shabrawi-Caelen, L. Cerroni, L. J. Medeiros, T. H.McCalmont, Hypopigmented Mycosis Fungoides: Fre-quent Expression of a CD8+ T-Cell Phenotype, TheAmerican Journal of Surgical Pathology, Vol. 26, No. 4,2002, pp. 450-457.doi:10.1097/00000478-200204000-00006

[12] A. Manzur and S. T. H. Zaid, Hypopigmented MycosisFungoides in a 10-Year-Old Boy, Dermatology OnlineJournal, Vol. 12, No. 6, 2006, p. 21.

http://dx.doi.org/10.1111/j.1525-1470.1997.tb00687.xhttp://dx.doi.org/10.1111/j.1525-1470.1997.tb00687.xhttp://dx.doi.org/10.1111/j.1365-2133.1982.tb11678.xhttp://dx.doi.org/10.1111/j.1365-2133.1982.tb11678.xhttp://dx.doi.org/10.1097/00000372-198608000-00009http://dx.doi.org/10.1097/00000372-198608000-00009http://dx.doi.org/10.4103/0019-5154.26932http://dx.doi.org/10.4103/0019-5154.26932http://dx.doi.org/10.1097/00000478-200204000-00006http://dx.doi.org/10.1097/00000478-200204000-00006http://dx.doi.org/10.1097/00000478-200204000-00006http://dx.doi.org/10.4103/0019-5154.26932http://dx.doi.org/10.1097/00000372-198608000-00009http://dx.doi.org/10.1111/j.1365-2133.1982.tb11678.xhttp://dx.doi.org/10.1111/j.1525-1470.1997.tb00687.x

Documents

Journal of Mycosis Fungoides